Uterine
sarcomas are rare and comprise only 3% of all
uterine cancers. Within the group of adult
soft tissue sarcomas, they account for ∼7% of new cases. They consist of several distinct histological subtypes like
leiomyosarcoma,
endometrial stromal tumors, undifferentiated
sarcomas, pure heterologous
sarcomas, and mixed epithelial and mesenchymal
tumors. Standard treatment in localized disease is abdominal
hysterectomy. Bilateral
salpingo-oophorectomy and
lymphadenectomy have no proven value in
leiomyosarcomas and high-grade undifferentiated
sarcomas. However, in
endometrial stromal tumors, given the hormonal reponsiveness of most
tumors,
salpingo-oophorectomy is generally recommended.
Carcinosarcomas are treated according to current recommendations for epithelial
uterine cancers. In
leiomyosarcomas, postoperative radiation does not improve both relapse-free and overall survival.
adjuvant chemotherapy seems to improve survival in the context of uncontrolled phase II trials. However, it is currently not considered standard of care in the absence of data from randomized trials. In contrast,
adjuvant chemotherapy does improve overall survival in
carcinosarcomas and is therefore considered standard of care. Systemic
therapy for advanced uterine
leiomyosarcomas, undifferentiated uterine
sarcomas, and heterologous
sarcomas is generally following the recommendations for adult
soft tissue sarcomas.
Endometrial stromal sarcomas are usually hormonal receptor positive, which allows endocrine
therapy in most cases.